Intensive Care Nursery House Staff Manual

Chronic Lung Disease (Bronchopulmonary Dysplasia)

was first described in 1967 as severe chronic lung disease (CLD) in preterm infants with
severe Respiratory Distress Syndrome (RDS) who received treatment with 100% O2, high
inspiratory ventilator pressures and no PEEP. With antenatal glucocorticoids, surfactant
treatment and improved ventilatory techniques, CLD has almost disappeared in larger
preterm infants and now affects very preterm infants with or without antecedent RDS.
DEFINITION: CLD is defined as a need for increased oxygen:
Infants <32 weeks gestation: oxygen requirement at 36 weeks gestational age (GA)
or at discharge (whichever comes first)
Infants 32 weeks GA: oxygen requirement at age >28 d or at discharge
(whichever comes first)
INCIDENCE of CLD is inversely related to birth weight and GA:
Birth weight (g) Incidence of CLD*
501-750 34% *UCSF 1998-2002
751-1,000 20%
1,001-1,250 5%
1,251-1,500 3%
PATHOLOGY includes areas of atelectasis and emphysema, hyperplasia of airway
epithelium and interstitial edema. Late changes include interstitial fibrosis and
hypertrophy of airway smooth muscle and pulmonary arteriolar musculature.
ETIOLOGICAL FACTORS include:
Lung immaturity with (a) susceptibility to damage from oxygen, barotrauma and
volutrauma, (b) surfactant deficiency and (c) immature antioxidant defenses.
Oxygen toxicity
Barotrauma and volutrauma
Pulmonary edema (excessive fluid administration, patent ductus arteriosus)
Inflammation (multiple associated biochemical changes)
RISK FACTORS include:
1. Maternal: Chorioamnionitis Abruptio placenta
No antenatal steroids Prenatal indomethacin
Intrauterine growth retardation
2. Neonatal:
Prematurity (<28 weeks GA) Birth weight <1,000 g
Male gender Low Apgar scores
Severity of RDS Air leaks
Patent ductus arteriosus Infection
Lung disease (CDH, pulmonary hypoplasia) Genetic factors
Risk: in Caucasians, in African-Americans
CLINICAL FEATURES:
Hypoxia due to V/Q mismatch work of breathing

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Copyright 2004 The Regents of the University of California
 Chronic Lung Disease

Abnormal chest radiograph airway resistance is late feature

Pulmonary hypertension Cor pulmonale (late)
PREVENTIVE MEASURES:
Minimize barotrauma & volutrauma by using low ventilator inspiratory pressures
and tidal volumes. Tolerate mild hypercarbia (PaCO2 55 mm Hg). Higher PEEP
(e.g., 6 cm H2O) may help prevent CLD. Early extubation and nasal CPAP may help,
but benefit has not been proven.
Minimize oxygen toxicity: Maintain O2 saturation between 85 and 92% in preterm
babies. Higher O2 saturation also risk of Retinopathy of Prematurity (ROP).
Careful attention to intake of fluid and Na+. Mild hyponatremia in small preterm
infants is common, is tolerated well and is not an indication to Na+ intake.
TREATMENT of ESTABLISHED CLD:
Adequate caloric intake (140-160 kcal/kg/d) because of work of breathing
After 36 weeks GA, maintain O2 saturation >95% to prevent pulmonary
hypertension and cor pulmonale. (Low risk of ROP after 36 weeks GA). Some
infants with CLD will require O2 therapy after discharge.
Restrict intake of fluid and Na+. Hyponatremia with serum sodium 125 mEq/L
should be treated with fluid restriction, not diuretics and administration of Na+.
Diuretics, especially furosemide. Do not use unless there already is restricted intake
of fluids and Na+. Side effects are common and include hypercalciuria (leading to
osteopenia, & nephrocalcinosis), metabolic alkalosis (due to Cl- loss) and
hypokalemia. Alternate day diuretics may be effective with fewer side effects.
Bronchodilators may be effective. Pulmonary function testing to document
bronchoconstriction prevents unnecessary use of these drugs. Tightness or
clamping down diagnosed by auscultation is often due to atelectasis, not
bronchoconstriction.
Steroids are almost never indicated in CLD, rarely have any lasting benefit and
significantly the risk of adverse neurologic outcome. Steroids should be used
only in very severe CLD (e.g., infant on high O2 and high ventilator settings who is
worsening). Other side effects include systemic hypertension, adrenal suppression,
infection, growth suppression and cardiac hypertrophy.
Infection prevention: Immunization against RSV infection (see section on
Immunizations, P. 71).
OUTCOME:
1. Mortality with severe CLD is ~25%. Main causes of death are cor pulmonale, lower
respiratory tract infection and sudden death.
2. Long term complications are common and include:
Respiratory: Recurrent infections, central apnea, bronchial hyper-reactivity
Cardiovascular: Pulmonary hypertension, cor pulmonale, bronchopulmonary shunts
Growth delay
GI: Feeding difficulties, GE reflux, aspiration
Associated conditions (but unlikely due to CLD): hearing loss, developmental delay,
cerebral palsy, intraventricular hemorrhage and white matter CNS damage.

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Copyright 2004 The Regents of the University of California